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T-cell-prolymphocytic leukemia (T-PLL) is a mature T-cell leukemia with aggressive behavior and predilection for blood, bone marrow, lymph nodes, liver, spleen, and skin involvement.[1] T-PLL is a rare leukemia, primarily affecting adults over the age of 30. It represents 2% of all small lymphocytic leukemias in adults.[2] Other names include: T-cell chronic lymphocytic leukemia, “Knobby” type of T-cell leukemia, T-prolymphocytic leukemia/T-cell lymphocytic leukemia[1]
Due to the systemic nature of this disease, leukemic cells can be found in peripheral blood, lymph nodes, bone marrow, spleen, liver, skin.[1]
It is postulated that the originating cell line for this disease is a mature (post-thymic) T-cell.[1]
Patients typically have systemic disease at presentation, including hepatosplenomegaly, generalized lymphadenopathy, and skin infiltrates.[1]
A high lymphocyte count (> 100 x 109/L)along with anemia and thrombocytopenia are common findings. HTLV-1 serologies are negative, and serum immunoglobins are within normal limits with no paraproteins present.[1]
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